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 Table of Contents     
CASE REPORT
Year : 2014  |  Volume : 29  |  Issue : 2  |  Page : 115-116  

Rare case of trilateral retinoblastoma with spinal canal drop metastasis detected with fluorine-18 fluorodeoxyglucose positronemission tomography/computed tomography imaging


Department of Nuclear Medicine and PET/CT, Comprehensive Cancer Care Centre, Kovai Medical Centre and Hospital Limited, Coimbatore, Tamil Nadu, India

Date of Web Publication9-Apr-2014

Correspondence Address:
MD (Nuclear Medicine) Koramadai Karuppusamy Kamaleshwaran
Department of Nuclear Medicine, PET/CT and Radionuclide Therapy, Comprehensive Cancer Care Centre, Kovai Medical Centre and Hospital Limited, Coimbatore - 641 014, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-3919.130303

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   Abstract 

Trilateral retinoblastoma (TRb) is a rare syndrome associating hereditary bilateral or unilateral retinoblastoma (Rb) with an intracranial neuroblastic tumor. The latter arises in the midline, most often in the pineal gland, less frequently in the suprasellar or parasellar region. The outcome is usually fatal because of secondary spinal dissemination. We report 10-year-old boy presented with a right eye proptosis and leukocoria, and the magnetic resonance imaging (MRI) showed right orbital mass lesion infiltrating optic nerve and diagnosis of retinoblastoma was made. He was referred for fluorodeoxyglucose-positron emission tomography/computed tomography (FDG PET/CT) to find out the extent of the disease. PET/CT showed abnormal FDG-uptake within right orbital mass lesion, suprasellar enhancing lesion and drop metastasis in the cervical spinal canal level. He was diagnosed as a case of TRb with spinal canal drop metastasis. He underwent chemotherapy and craniospinal irradiation.

Keywords: Drop metastasis, fluorodeoxyglucose-positron emission tomography/computed tomography intracranial neuroblastic tumor, trilateral retinoblastoma


How to cite this article:
Kamaleshwaran KK, Shibu DK, Mohanan V, Shinto AS. Rare case of trilateral retinoblastoma with spinal canal drop metastasis detected with fluorine-18 fluorodeoxyglucose positronemission tomography/computed tomography imaging. Indian J Nucl Med 2014;29:115-6

How to cite this URL:
Kamaleshwaran KK, Shibu DK, Mohanan V, Shinto AS. Rare case of trilateral retinoblastoma with spinal canal drop metastasis detected with fluorine-18 fluorodeoxyglucose positronemission tomography/computed tomography imaging. Indian J Nucl Med [serial online] 2014 [cited 2019 May 19];29:115-6. Available from: http://www.ijnm.in/text.asp?2014/29/2/115/130303


   Introduction Top


Trilateral retinoblastoma (TRb) is a rare disease associating intraocular retinoblastoma with intracranial primitive neuroectodermal tumor. [1] Treatment is difficult, and prognosis is poor. TRb can occur with both familial and sporadic forms of retinoblastoma. [2] To the best of our knowledge, this is the first case report of positron emission tomography/computed tomography ( PET/CT) imaging features in TRb with suprasellar mass and drop metastasis.


   Case Report Top


A 10-year-old boy presented with a right eye proptosis and leukocoria. He underwent magnetic resonance imaging (MRI) which showed right orbital mass lesion infiltrating optic nerve, and diagnosis of retinoblastoma was made. He was referred for fluorodeoxyglucose (FDG) PET/CT to find out the extent of the disease. Whole body contrast enhanced PET/CT [Figure 1]a showed abnormal FDG-uptake within right orbital mass lesion [Figure 1]b and suprasellar region [Figure 1]c, sagittal fused-PET/CT showing enhancing lesion in the cervical spinal canal level [[Figure 1]d, arrows]. He was diagnosed as a case of TRb with spinal drop metastasis. He underwent chemotherapy and craniospinal irradiation.
Figure 1: Whole body contrast enhanced fluorodeoxyglucose-positron emission tomography/computed tomography (FDG PET/CT) maximum intensity projection image (a) Axial fused-PET/CT showed intense uptake in the soft tissue lesion in the right orbit (b) suprasellar mass lesion and (c) sagittal fused-PET/CT showing drop metastasis in cervical spinal canal level (d, arrows). Also, physiological brown fat uptake noted in bilateral cervical, supraclavicular, and paravetrbral locations

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   Discussion Top


TRb is a disease associating unilateral or bilateral Rb with an intracranial midline primitive neuroectodermal tumor, which usually arises in the pineal gland (77%). [1] The risk of developing TRb in Rb patients is less than 0.5% for sporadic unilateral disease, 5-13% in sporadic bilateral disease, and 5-15% in familial bilateral Rb. [2] Patients with TRb were frequently present with signs of intracranial hypertension. [3] The unusual presence of leptomeningeal dissemination at diagnosis raises the question whether the suprasellar tumor could be a metastasis. Its strict midline location, the absence of continuity and intraorbital extension on MRI, the absence of diffuse intracerebral nodules, are strong factors against metastasis as well as the close relationship to Rb. [4] Marcus et al.,[5] who described in their pathologic review on 80 cases of TRb, 9 suprasellar and parasellar masses with hypointense images on MRI and significant enhancement of gadolinium. In 36 cases, intracranial tumor spread was documented at autopsy and comprised seeding along the spinal cord and canal, diffuse meningeal, ependymal and subependymal involvement, invasion into brain parenchyma, and optic nerve invasion. CT or MRI of the orbit and brain are the standard imaging modalities used for diagnosing and evaluating disease extent in retinoblastoma. [6] To the best of our knowledge, the role of 18F-FDG PET/CT in TRb has not been reported in the published literature. There has been previous report on the use of 18F-FDG PET alone without CT in 4 patients with retinoblastoma [7] and Radhakrishnan et al.,[8] described the role of PET/CT in staging and evaluation of treatment response after three cycles of chemotherapy in locally advanced retinoblastoma. This is the first case of identifying TRb with drop metastasis in PET/CT. Recognizing and understanding the clinical findings may determine the overall management of the patients. Treatment of these patients is very difficult and prognosis is poor despite a multimodality approach, as most of them die of leptomeningeal dissemination. [9]

 
   References Top

1.Kivela T. Trilateral retinoblastoma: A meta-analysis of hereditary retinoblastoma associated with primary ectopic intracranial retinoblastoma. J Clin Oncol 1999;17:1829-37.  Back to cited text no. 1
    
2.De Potter P, Shields CL, Shields JA. Clinical variations of trilateral retinoblastoma: A report of 13 cases. J Pediatr Ophthalmol Strabismus 1994;31:26-31.  Back to cited text no. 2
    
3.Provenzale JM, Gururangan S, Klintworth G. Trilateral retinoblastoma: Clinical and radiological progression. Am J Radiology 2004;183:505-11.  Back to cited text no. 3
    
4.Paulino AC. Trilateral retinoblastoma. Cancer 1999;86:135-41.  Back to cited text no. 4
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5.Marcus DM, Brooks SE, Leff G, McCormick R, Thompson T, Anfinson S, et al. Trilateral retinoblastoma: Insights into histogenesis and management. Surv Ophthalmol 1998;43:59-70.  Back to cited text no. 5
    
6.Chintagumpala M, Chevez-Barrios P, Paysse EA, Plon SE, Hurwitz R. Retinoblastoma: Review of current management. Oncologist 2007;12:1237-46.  Back to cited text no. 6
    
7.Moll AC, Hoekstra OS, Imhof SM, Comans EF, Schouten-van Meeteren AY, van der Valk P, et al. Fluorine-18 fluorodeoxyglucose positron emission tomography (PET) to detect vital retinoblastoma in the eye: Preliminary experience. Ophthalmic Genet 2004;25:31-5.  Back to cited text no. 7
    
8.Radhakrishnan V, Kumar R, Malhotra A, Bakhshi S. Role of PET/CT in staging and evaluation of treatment response after 3 cycles of chemotherapy in locally advanced retinoblastoma: A prospective study. J Nucl Med 2012;53:191-8.  Back to cited text no. 8
    
9.Rodriguez-Galindo C, Wilson MW, Haik BG, Lipson MJ, Cain A, Merchant TE, et al. Treatment of metastatic retinoblastoma. Ophthalmology 2003;110:1237-40.  Back to cited text no. 9
    


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